636 Successful treatment of pachyonychia congenita with simvastatin

Background: Pachyonychia congenita (PC) is a rare keratinization disorder with around 1,000-10,000 cases reported worldwide. The mutation involves: KRT6A, KRT6B, KRTC6C, KRT16 or KRT17. Patients present severe plantar pain, palmoplantar keratoderma underlying blisters, and variable hypertrophic nail dystrophy [1]. Visual Analogue Scale (VAS) measures pain intensity. VAS consists of two endpoints representing 0 (‘no pain’) 10 (‘pain as bad it could possibly be). Case presentation: We case 45-year-old male patient who was diagnosed pachyonychia at the age 16. He presented hypertrophic, yellowish plates, painful thickened, lichenified in addition to fissuring callosities over heels. previously managed moisturizers, urea 10%-40%, salicylic acid topical ointment, betamethasone without improvement his condition. started on simvastatin when he returned 9 months later, showed great score ( from 8 2, before after treatment, respectively). Following simvastatin, demonstrated marked keratoderma, ungual dystrophy, hyperkeratosis, reduction an overall quality life DLQI 26 3 Discussion: As now no curative therapy available for PC[3]. Statins work via inhibition expression keratin genes by inhibiting STAT1 expression. responsible constitutive KRT6A promoter activity [4]. Here we fifth report worldwide successfully treated statin. Our demonstrates second PC successful simvastatin. Conclusion: Simvastatin provides promising new therapeutic approach address reduce hyperkeratosis associated PC. In genetic disease limited approaches, statins provide safe option improve patient's life.